Family House Diaries: Breathing Normally with New Lungs

For the first time in his life, Justin Hughes, a 26-year-old cystic fibrosis patient from Ash, N.C., is able to breathe normally and sleep without coughing thanks to a lung transplant at UNC Hospitals.

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Justin Hughes, right, with his father, Timmy, on the porch at SECU Family House.

For the first time in his 26 years, Justin Hughes is able to breathe normally and sleep without coughing thanks to a lung transplant at UNC Hospitals.

Born with cystic fibrosis (CF) and its related digestive system issues, Justin made his first trip to UNC Hospitals as a sickly three-hour-old infant.

“They flew me in from Whiteville on an Army helicopter, did some exploratory surgery, put me back together, diagnosed the CF and kept me here for eight weeks,” said Justin of Ash, N.C., a tiny crossroads community near the Columbus-Brunswick county line.

That early diagnosis of the inherited disease that causes abnormally thick, sticky mucus to build up in the breathing passages and in the pancreas, was key to Justin’s survival to adulthood, said James Yankaskas, M.D., professor of medicine at the UNC School of Medicine and co-director of the adult cystic fibrosis program at UNC.

“When cystic fibrosis was first defined in 1938, fewer than half of all patients survived one year,” said Yankaskas. “By 1980 – a few years before Justin was born – median survival was 18 years. By 2008, the median survival was over 37 years, and 47 percent of all CF patients were over 18 years of age.

“Improvements have been realized thanks to early diagnoses and research into the specialized care needed for the lungs, the comprehensive management of patients’ nutritional needs and how complications of the disease are identified and managed,” Yankaskas said.

Despite his illness, Justin considered himself “just a normal guy” as a kid and stayed out of the hospital until he was an early teenager. In high school, he enjoyed auto shop and welding classes and upon graduation, joined his father, Timmy, in the family-owned salvage yard and tire business. He was able to keep up with his favorite pastimes – rebuilding cars and motorcycles, hunting and fishing, the latter in fresh water only. “There are big things I can’t see in the ocean, like sharks,” Justin said.

Justin was added to the lung transplant list in 2000, at a time when patients moved up to the top spot based on how long they had been on the list. He remained inactive on the list until October 2009 when his condition worsened and new guidelines propelled the sickest patients to the top of the list.

“I had already had two surgeries last year – gallbladder removal in January and surgery for an abdominal obstruction in August – both complications from cystic fibrosis,” Justin said.

The first call that new lungs were available came on Oct. 12. Justin’s already-high-anxiety went into overdrive at the prospect of the long-awaited surgery, only to plummet when it was discovered that the prospective donor had a blood infection that made the lungs unusable for transplant.

The second call came in December when Justin was already at UNC Hospitals for treatment. The “false alarm” in October had prepared him for what to expect emotionally, and he was ready – happy even – during the 90-minute prep time before surgery. “Maybe there should always be a dry-run so patients and families know what to expect,” Justin mused.

On Dec. 12, Justin received new lungs during nearly eight hours of surgery. An incision from one armpit, down across his chest, just under the front of the rib cage and up to the opposite armpit, allowed Justin’s medical team to remove his sick lungs and slip in the new ones.

Justin was on a ventilator for a few days and wore a mask that forced air into his lungs when he inhaled. By the end of February, the first two bronchoscopies that look for signs of organ rejection came back clean.

“While a lung transplant is not a cure for cystic fibrosis, the new lungs will allow Justin to be more physically active and get on with the business of living,” Dr. Yankaskas said, adding that as with any transplant there are concerns about infection, rejection and other complications, some of which are known side effects of required medications.

“Justin traded a progressive, life-threatening lung condition for another set of issues,” Yankaskas said. “But it was a risk worth taking. He’s following a good clinical course and is doing well.”

On Jan. 7, Justin was released to SECU Family House, where family members had been staying while he was hospitalized. SECU Family House, a 40-bedroom hospital hospitality house minutes from UNC Hospitals, provides comfortable, convenient and affordable housing for adult patients undergoing treatment for critical illness and trauma and their family member caregivers.

Justin’s father, step-mother, mother and grandmothers rotate staying with him at SECU Family House to keep him company during the mandatory 100-day post-transplant stay in Chapel Hill and to accompany him to three-times-a-week physical therapy and routine post-transplant tests.

“Family House has been a godsend,” said Linda Bartlett, Justin’s step-mother. “We were told to make ourselves at home and we have. We’ve been able to keep working back home and have family here to support Justin. Everyone at Family House is so helpful, and we’ve made some really close friends here who are going through some really bad things.”

Justin and his family have always trusted UNC Hospitals with his care, and they don’t intend for that to change after heading home at the end of March.

“We have a comfortable relationship with my medical team, and they are all thoroughly familiar with my care,” Justin said, adding that he and his family were touched that nurses, respiratory therapists and other healthcare professionals who have cared for him and grown to know him through his lifetime of care at UNC Hospitals stopped by to see him after the transplant.

“It was really good to look up and see all those friendly faces,” he said.